Manifestation And Long-term Prognosis Of Microscopic Polyangiitis Patients With Pulmonary Involvement

Objective:To analyze the clinical characteristics and long-term prognosis of patients with microscopic polyangiitis with pulmonary involvement.Methodology:196 MPA patients with renal involvement were enrolled in the study.The median serum creatinine(SCr)was 344.8(176.8-556.9)umol/L,70(35.7%)of them needed renal replacement therapy at diagnosis.The prognosis and risk factor of MPA with pulmonary involvement were retrospectively studied.Survival Curve was estimated by Kaplan-Meier methods,and Multivariate Cox regression analysis is applied to probe into the correlation between clinical features as well as pathological parameters and long-term human survival outcome.Results:With a mean follow-up time of 38.9±42.0 months,20 patients died,81(41.3%)developed end stage renal disease(ESRD).The pulmonary involvements were observed in 111 MPA patients,their main clinical symptoms included hemoptysis and cough;CT or X-ray manifestations included patchy,funicular shadow and pleural thickening.The age,proportion of male patients and smokers,BVAS score,serum creatinine and MPO-ANCA level of pulmonary involvement group were apparently higher than those of non-pulmonary involvement group(P<0.05).The 1-year complete remission rate was 13.5%and 21.2%(P=0.155)in patients with and without pulmonary involvement groups;while their ineffective treatment proportion was respectively 42.3%and 35.3%(P=0.317).Patient survival rate in pulmonary involvement group was significantly lower(P=0.013);renal survival was not differed between two groups.Cox multivariate analysis indicated that lung involvement and dialysis dependent on admission were significant negative prognostic factors for patients survival.Conclusion:The vasculitis of older MPA patients with lung damage was more active.Lung involvement was an independent risk factor for human survival.